Lymphoproliferative Syndrome 1 C3552634
Lymphoproliferative syndrome type 1 is an immune system disorder where immune cells grow uncontrollably. It often develops after Epstein-Barr virus infection and can cause life-threatening complications. Treatment may include immunosuppressive medications, chemotherapy, and in some cases, bone marrow transplantation.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Disseminated EBV infection
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent respiratory infections
(immunologic)Persistent lymphadenopathy
(immunologic)Autoimmune hemolytic anemia
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin replacement therapy
Regular intravenous or subcutaneous immunoglobulin infusions to compensate for antibody deficiencies common in XLP1, reducing susceptibility to infections and autoimmune complications.
Allogeneic hematopoietic stem cell transplant
Curative approach involving transplantation of healthy donor stem cells to replace defective immune cells, addressing the underlying genetic immune dysfunction in XLP1.
Additional treatment options
Rituximab therapy
Anti-CD20 monoclonal antibody targeting B-cells to prevent or treat EBV-driven lymphoproliferation, often used as bridge therapy before definitive transplant.
medicationAntiviral prophylaxis
Prophylactic antiviral medications such as acyclovir or valganciclovir to prevent or control Epstein-Barr virus reactivation, which poses particular risk in XLP1 patients.
medicationImmunological and virological monitoring
Regular monitoring of immunoglobulin levels, lymphocyte subsets, EBV viral load, and organ function to guide therapy adjustments and detect complications early.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Lymphoproliferative Syndrome 1 C3552634?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy